More than 3000 mutations have been clinically identified in the Fibrillin-1 (FBN1) gene in humans. These mutations have been associated with a variety of conditions, including type I fibrillinopathies, Marfan syndrome, MASS syndrome, isolated ectopia lentis syndrome, thoracic aortic aneurysms, Weill-Marchesani syndrome, geleophysic and acromicric dysplasia, stiff skin syndrome, and neonatal progeroid syndrome with congenital lipodystrophy (NPSCL). Currently available transgenic non-human mammals engineered to have FBN1 mutations do not adequately reflect the symptoms of NPSCL.